Buzzwords - pathology

Question 1

Focal segmental glomerulosclerosis

Answer 1

LM: segmental sclerosis and hyalinosis
IF -
EM: effacement of foot process

Nephrotic
Af Am/Hispanics

Question 2

Membranous Nephropathy

Answer 2

LM: diffuse capillary and GBM THICKENING
IF: granular - immune complex deposition
EM: “SPIKE AND DOME”

Nephrotic
Causasian adults
IgG ab to phospholipase A2

Question 3

Minimal Change Disease

Answer 3

LM: normal
IF: -
EM: effacement of foot process

Nephrotic
Children
Abn T cell production

Question 4

Amylodosis - renal

Answer 4

LM: congo red stain - apple green birefringence under polarized light

Nephrotic

Question 5

Membrano-proliferative glomerulonephritis

Answer 5

Type 1: Immune complex deposits with IF; TRAM TRACK d/t GBM splitting (HBV/HCV)

Type 2: Intramembranous IC deposits - dense deposits - associated with C3 NEPHRITIC FACTOR

Nephritic & nephrotic
mesangial proliferiation

Question 6

Diabetic glomerulonephropathy

Answer 6

LM: Kimmelsteil Wilson Lesion

Nonenzymatic glycosylation of GBM

Eosinophilic
Nephrotic

Question 7

Acute PSGN

Answer 7

LM: glomeruli enlarged/hypercellular
IF: STARRY SKY - d/t IgG, IgM, C3 deposition
EM: SUBEPITHELIUM IMMUNE COMPLEX (IC) HUMPS

Nephritic
T3H

peripheral & periorbital edema, COLA COLORED URINE, HTN

Increased anti-DNase B titers
Decreased complement

Question 8

Rapidly progressive (crescentic) glomerulonephritis (RPGN)

Answer 8

LM & IF: CRESCENT moon - FIBRIN & PLASMA PROTEINS

Goodpasture (T2H - pulm & renal - ab to GBM)
Granulomatosis with polyantigitis (Wegener) - C-ANCA (upper/lower airway, renal)
Microscopic polyangitis - pANCA

Question 9

Diffuse proliferative glomerulonephritis

Answer 9

SLE/MPGN

most common cause of death in LUPUS

LM: wire loops capillaries
EM: subendothelial and intramembranous IgG based IC w/ C3 deposition
IF: granular

Question 10

IgA nephropathy (Berger dz)

Answer 10

LM - mesangial proliferation
EM: mesangial IC deposits
IF: IgA based IC deposits in mesangium

HSP

Question 11

Alport syndrome

Answer 11

IV collagen mutation

Ear, eye, family HX
“Can’t see, can’t pee, can’t hear a bee”

Question 12

ADPKD

Answer 12

Autosomal dominant polycystic kidney dz

Bilateral, massive enlargement of kidneys d/t multiple large cysts
85% - PKD1 (Chromosome 16)
15% - PKD2 (Chromosome 4)

Question 13

Familial adenomatous polyposis

Answer 13

Adenomatous polyps after pubery
APC mutation on chromosome 5

5 letters in polyp

Question 14

Familial hypercholesterolemia

Answer 14

Elevated LDL - defective or absent LDL receptor

Question 15

Hereditary hemorrhagic telangectasia

Answer 15

Inherited disorder of blood vessels

Telangectasia, recurrent epistaxis, skin disoloration, AVM, GI bleeding, hematuria,

Osler-Weber-Rendu

Question 16

Hereditary sperocytosis

Answer 16

Spherocyte erythrocytes - defective spectrin or ankrin

Hemolytic anemia, increased MCHC

TX: splenectomy

Question 17

Huntington Dz

Answer 17

Depression, progressive dementia, choreiform movements, caudate atrophy

decreased levels GABA & Ach in brain
Chromosome 4 - CAG repeat

Question 18

Marfan s;yndrome

Answer 18

Fibrillin 1 gene mutation

CT disorder - skeleton, heart, eyes

Tall w/ long extremities, pectus excavatum, hypermobile joints, & long tapering fingers and toes

Cystic medial necrosis of aorta - aortic incompetence and dissecting aortic aneurysm, floppy mitral valve
Subluxation of lens (up and temporal)

Question 19

Mutliple endocrine neoplasia

Answer 19

1, 2A, 2B
Famililal tumors of endocrine glands
pancreas, parathyroid, pituitary, thyroid, adrenal medulla
2A & 2B - ret gene

Question 20

Neurofibromatosis type 1

Von Recklinghausen Dz

Answer 20

Neurocutaneous - cafe au lait spots and cutaneous neurofibromas

AD - 100% penetrance but variable expression

NF1 gene on chromosome 17

Question 21

NF 2

Answer 21

Bilateral schwannomas
Juvenile cataracts
Meningiomas
Ependymomas

NF2 on chromosome 22

Question 22

Tuberous sclerosis

Answer 22

Neurocutaneous

Benign hamartomas

Question 23

von hippel lindau

Answer 23

numerous tumors (benign and malignant) 
deletion of VHL gene (tumor suppressor) on chromosome 3